Case Presentation: A 64 year old male suffered from gradual onset fatigue and change in mentation over the course of a month. His symptoms then progressively worsened as he became confused and developed intermittent headaches. Past medical history includes pulmonary sarcoidosis, treated with 20mg of prednisone daily.
On presentation, the patient had no neck stiffness. He was afebrile, with stable vital signs. Neurological exam was non-focal. CBC and electrolytes were unremarkable. Chest x-ray showed possible right lower lobe opacity. He was started on antibiotics for possible pneumonia and admitted.
During the hospital stay he did not improve. Head CT was unremarkable. Neurosarcoidosis was suspected, and a lumbar puncture was performed. CSF showed a modest lymphocytic pleocytosis and raised protein level. A cryptococcal antigen was obtained and came back positive at a titer of 1:1280. The patient was subsequently diagnosed with cryptococcal meningitis (CM) and started on Amphotericin and Flucytosine. Mental status improved and he was discharged home in a stable condition.
Discussion: Sarcoidosis is a systemic disease of unknown etiology, characterized by the formation of noncaseating granulomas in various organs. Affected patients are also predisposed to CM. In fact, sarcoidosis is an independent risk factor for invasive cryptococcal infections which carry a high mortality rate if not recognized and treated early. Classically, CM presents with subacute to chronic progressive headache, lethargy, and altered mental status. Diagnosis is made by obtaining CSF and testing for the Cryptococcal antigen. MRI imaging may show characteristic changes consistent with the infection, however is usually not necessary for diagnosis. Treatment is prolonged and involves induction with amphotericin B and flucytosine for two weeks followed by high dose fluconazole for eight weeks, then maintenance therapy with low dose fluconazole for one year.
Conclusions: CM in sarcoid patients often presents with non-specific symptoms, and should be excluded when neurological disturbances are present due to the high morbidity and mortality. Of concern is a misdiagnosis of neurosarcoidosis which can result in considerable treatment delay and worse outcome. Neurosarcoidosis and CM often present with chronic meningitis symptoms and can have overlapping clinical and laboratory features. CSF cryptococcal antigen is the most specific diagnostic test; hence it should be done whenever this diagnosis is suspected.